Case Report
Nasosinusal Lymphoma of T Natural Killer Cells:
Case Report
Linfoma Nasossinusal de Células T Natural Killer: Relato de Caso
Victor Labres da Silva Castro*, João Batista Ferreira**, Valeriana de Castro Guimarães***,
Gustavo Vasconcelos Nery****, Tiago Fernando Côrrea Aires*****, Wilder Alves****.
* Resident Physician in Otorhinolaryngology.
** Postdoctoral Degree in Otorhinolaryngology. Assistant Professor of the Surgery Department of the Medical School - UFG. Chief of the Otorhinolaryngology Clinic
of the Clinical Hospital - UFG.
*** In course for Doctoral Degree in Health Sciences UFG. Phonoaudiologist of the Clinical Hospital - UFG.
**** Otorhinolaryngologist of the Clinical Hospital - UFG.
***** Resident Physician in Otorhinolaryngology of the Clinical Hospital - UFG.
Institution:
Hospital das Clínicas da Universidade Federal de Goiás.
Goiânia / GO - Brazil.
Mail address: Victor Labres da Silva Castro - Hospital das Clínicas da Universidade Federal do Goiás - Primeira Avenida, s/n - Setor Leste Universitário - Goiânia / GO
- Brazil - Zip code: 74605-020 - E-mail: vlabres@hotmail.com
Article received on June 16, 2009. Approved on July 12, 2009.
SUMMARY
Introduction:
Objective:
Case Report:
Final Comments:
Keywords:
The primary nasal lymphoma is an uncommon extranodal tumor and represents 0.44% of all Extranodal
lymphomas in this region. The primary nasal lymphoma derives from the T-lineage in nearly 75% of
the cases.
To describe a case of nasosinusal lymphoma of T Natural Killer cells, attended in the Clinical Hospital
of the Federal University of Goiás.
48-year-old female patient with diffuse tumefaction in the left hemiface of firm-elastic consistency and
painful upon digital compression. Face sinuses tomography identified a total maxillary veiling to the
left and some posterior ethmoidal cells. With the diagnostic hypothesis of a tumor affection, we opted
for the surgical removal via a transmaxillary approach and the material was sent for biopsy. The
histopathological exam diagnosed a highly necrotic tumor of angiocentric pattern, polymorphic and
atypical lymphoid population (T /NK Lymphoma); with the prognosis, the patient was submitted to
chemical therapy with total regression of the facial edema.
The otorhinolaryngologist must be attentive as regards the existence of lymphomas among the nasosinusal
diseases, because the early diagnosis improves the survival as it prevents metastases, growth and local
destruction.
T-NK cells extranodal lymphoma, non-Hodgkin lymphoma, lymphoma, cells lymphoma.
RESUMO
Introdução:
Objetivo:
Relato do Caso:
Comentários Finais:
Palavras-chave:
O linfoma nasal primário é um tumor extranodal raro e representa 0,44% de todos os linfomas extranodais
nessa localização. O linfoma nasal primário deriva da linhagem T em torno de 75% dos casos.
Descrever um caso de Linfoma nasossinusal de células T Natural Killer, atendido no Hospital das
Clínicas da Universidade Federal de Goiás.
Paciente de 48 anos, sexo feminino, apresentando tumefação difusa na hemiface esquerda, de consistência firme-elástica e dolorosa a compressão digital. Tomografia dos seios da face identificou um
velamento maxilar total à esquerda e de algumas células etmoidais posteriores. Com a hipótese diagnóstica
de uma afecção tumoral, optou-se por remoção cirúrgica via transmaxilar, sendo encaminhado o
material para biopsia. O exame histopatológico diagnosticou um tumor altamente necrótico padrão
angiocêntrico, população linfoide polimórfica e atípica (Linfoma T/NK), diante do diagnóstico a paciente
foi submetida à quimioterapia com regressão total do edema facial.
O otorrinolaringologista deve estar atento para a existência dos linfomas entre as doenças nasossinusais,
pois o diagnóstico precoce melhora a sobrevida na medida em que previne metástases, crescimento
e destruição local.
linfoma extranodal de células T-NK, linfoma não Hodgkin, linfoma; linfoma de células T.
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Castro et al.
INTRODUCTION
The primary nasal lymphoma is an uncommon
extranodal tumor and represents 0.44% of all Extranodal
lymphomas in this region. Among the extranodal tumors,
the non-Hodgkin lymphomas are divided into B and T
cells neoplasms and T Natural Killer cells lymphomas - T/
NK, and are frequent in the east countries, but uncommon
in the west population. Approximately 75% of the cases
of primary nasal lymphoma result from T cell-line (1, 2).
The
phenotype
is
determined
by
immunoperoxidase with several monoclonal antibodies.
The CD 56 expression (molecule of neural cellular adhesion,
Natural Killer cell marker) is uncommon among the
lymphomas, but it defines the lymphoma of T cells/
Natural Killer (3).
Figure 1. Left hemiface edema. Firm and red consistency. HC/
UFG 2007.
The T/NK cells lymphoma may manifest in any age
group, with prevalence of the male sex and older groups
and voluminous tumor; factors related to low survival. This
neoplasm may disseminate to other extranodal sites, like
skin, subcutaneous tissue, gastrointestinal tract, testicles
and others (2, 4).
In this article we propose the presentation of a
case whose relevance is the fact it configures an
uncommon case in the otorhinolaryngology service and
other fields.
This report is intended to describe a case of
nasosinusal lymphoma of T Natural Killer cells, seen in the
Clinical Hospital of the Federal University of Goiás.
CASE REPORT
Patient aged 48 years old, female sex, from Goiânia
- Goiás, sought the Emergency Service of
Otorhinolaryngology of the Clinical Hospital of the Federal
University of Goiás in November 2007, with the following
clinical profile: painful and progressive edema in the left
hemiface for one month with purulent rhinorrhea without
recovery with antibiotics. Also presenting a slight loss of
weight and night sudoresis since the beginning of the
disease. Without other otorhinolaryngological and local
symptoms.
Upon physical exam, the patient was in good
general state with normal vital signs. In the cervical facial
inspection and palpation, we identified diffuse tumefaction
in the left hemiface of firm-elastic consistency and painful
upon digital compression. Without alterations in the region
of the neck (Figure 1).
Figure 2. CT of facial sinuses. Axial cut in gap of soft parts
confirms the presence of a material with soft parts density and
occupies the maxillary sinuses. There is an extension of the
process in the left side by the anterior and medial walls of the
maxillary sinus with a small stain of the pre-maxillary fat. HC/
UFG 2007.
Upon clinical otorhinolaryngological evaluation, the
oropharyngoscopy and otoscopy showed no alterations.
The anterior rhinopharyngoscopy showed purulent
secretion in the left middle meatus.
The nasopharyngeal laryngoscopy described a
secretion in the left middle meatus with a covering across
the mucosa of edemaciated and obstructive appearance.
Right nasal cavity did not present alterations.
The computed tomography of the facial sinuses
revealed a total left maxillary veiling with affection of
posterior ethmoidal cells (Figure 2 and 3). Suggestion of
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DISCUSSION
The interest in the description of this case is
confirmed with the rarity with which the disease occurs as
well as its development and conclusion.
Figure 3. CT of facial sinuses. Coronal cut in osseous gap does
not show evidences of parietal osseous lesion. HC/UFG 2007.
pre-maxillary inflammatory process (cellulitis) at the left
side with increase of the left hemiface volume.
With this clinical profile, the patient was admitted.
We requested a report from the hematology staff and lab
exams like: Blood-count, coagulogram, blood sugar and
creatinine whose results were normal and negative antiHIV 1 and 2 sorology.
With diagnostic hypothesis of tumor lesion original
from the left maxillary sinus, we opted for incisional
biopsy through transmaxillary surgical approach to obtain
fragments of the mass and further anatomopathological
analysis.
The histopathological exam diagnosed a highly
necrotic tumor of angiocentric pattern, polymorphic and
atypical lymphoid population (T/NK Lymphoma). Markers:
CD3+, CD30+, CD5+, EBV-, CD56+, CD43-, ALC+, AE1AE3, CD45Re+, CD20-.
The total thorax and abdomen computed
tomography showed no alterations. The biopsy presented
a normocellular bone marrow for the age, with occupation
rate of 50%.
With the diagnosis of NK cells lymphoma of the left
maxillary sinus and absence of metastasis a
chemotherapeutic treatment was started with total regression
of the facial edema and strong recovery of the profile.
During a one-year follow-up, the clinical recovery was
maintained.
In the case described, the patient had an affection
of the left hemiface and paranasal sinuses. The literature
describes the gastrointestinal tract is the most common
region for appearing of the extranodal lymphoma,
followed by the region of the head and neck. In this
region, the most affected sites are the nasopharynx,
tonsils and base of the tongue. Other structures like the
paranasal sinuses, orbit and the salivary glands may be
affected. However, the involvement of the oral cavity is
uncommon (5, 6, 7).
The non-Hodgkin Lymphoma frequently affects
adults aged between 40 and 80 years. There is a clear
relation of incidence between the non-Hodgkin lymphoma
with positive HIV sorology. Positive HIV individuals have
60 times more risks than the general population. Studies
show that 3% of the people infected with HIV develop
lymphomas (8, 9). Contrarily in the case presented there
was negative Elisa serology for HIV 1 and 2.
The nasosinusal neoplasm diagnosis is late many
times due to its possible lowly apparent oligosymptomatic
manifestation. Clinical manifestations like: facial pain,
edema, epistaxis, purulent secretion, odontogenic pain,
nasal obstruction, sinusitis, sinus cutaneous fistula, necrosis,
ulceration and septum perforation, etc., may be present
and sometimes are followed by fever and weight loss (2,
3). In the case reported there was facial edema with
redness, night sudoresis, purulent rhinorrhea and weight
loss. The clinical profile developed by the patient is
compliant to the literature reports and suggests the disease’s
diagnosis.
The CD-56 is the specific marker for Natural Killer
Lymphocyte and the T/NK lymphoma composes a new
terminology for a number of eponyms that named this
disease before. This lymphoma offers a limited prognosis
and the conflicting survival statistics of 9% in three years
until 46 to 63% in five years (10). This marker was present
in the immunohistochemical study of the material extracted
from the patient’s maxillary sinus.
FINAL COMMENTS
Taking the recurrent process into account, the
patient remains in follow up with the hospital’s
otorhinolaryngology and hematology staffs for attention to
the treatment.
The otorhinolaryngologist should beware of the
existence of lymphomas among the nasosinusal diseases.
The nasosinusal lymphoma of T/NK cells is an uncommon
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Nasosinusal lymphoma of T natural killer cells: Case report.
extranodal lymphoma, however it must be considered in
the differential diagnosis of the tumors of this region.
The joint performance with the hematologist is
important for the treatment and conduction of this disease
because chemotherapeutic intervention is required.
The professional must be attentive with unspecific
and dragged profiles, once the identification and the early
diagnosis improve the survival as it prevents metastases,
growth and local destruction.
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